Case report
Clinical, neuropathological and radiological evidence for a rare complication of rituximab therapy

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Highlights

  • Anti CD 20 therapy may result in unusual infections.

  • We report enterovirus myofasciitis with documented pathology for the first time.

  • Increased susceptibility to rare viruses should be considered in treated patients.

  • Echovirus related muscle disease manifests as myofasciitis not dermatomyositis.

  • Successful treatment can be considered as discussed in this report.

Abstract

We report a rare case of myofasciitis and meningitis with deafness caused by systemic enterovirus infection in the setting of hypogammaglobulinaemia induced by rituximab. Whilst effective and generally safe, anti- CD 20 antibody therapy is increasingly recognised to result in unusual infectious complications to be considered in a treated patient presenting with neurological symptoms. These cases may pose diagnostic difficulties and can have atypical presentations. We present this rare complication of rituximab therapy, with histopathological confirmation of myofasciitis. In the older literature, enterovirus associated myofasciitis may have erroneously been termed dermatomyositis and we review the literature to demonstrate this important nosological point.

Introduction

Anti CD 20 therapy may result in unusual infections with atypical presentations, posing diagnostic difficulties. We highlight the long term immunomodulatory effects of rituximab by reporting the rare complication of myofasciitis, with clear documentation of the pathology of enterovirus myofasciitis for the first time.

Section snippets

Case report

A 36-year-old man was treated four years into his diagnosis of a grade 1 follicular lymphoma, with six cycles of R-CHOP chemotherapy. He achieved a complete metabolic remission on re-staging positron emission tomography (PET) scanning. Chemotherapy was complicated by two episodes of neutropenic sepsis and mild iatrogenic deafness with vestibular failure from aminoglycoside use. One year following treatment, whilst on maintenance therapy with rituximab for three months, he developed myalgia,

Discussion

Chronic ‘myositis’ and meningitis due to echovirus infection is documented in patients with primary immunoglobulin deficiencies [1], [2], [3]. A number of enteroviral strains have been isolated from the CSF but not in muscle, as in this case. It is hypothesised that the role of enteroviruses in the pathogenesis of inflammatory muscle disease may be related to pathways of autoimmunity following acute infection, without persisting viral replication. Many early cases have been described to have an

Acknowledgments

We thank Dr Tim Diss, Clinical Scientist at University College Hospital London for PCR analysis and Dr Zane Jaunmuktane, Specialty Registrar in Neuropathology, Division of Neuropathology, National Hospital for Neurology and Neurosurgery, for slide scanning and image preparation. We thank the UCLH/UCL CBRC for their support.

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